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Anais Brasileiros de Dermatologia

Print version ISSN 0365-0596On-line version ISSN 1806-4841

Abstract

SALA, Ana Carolina Bulhões et al. Scleromyxedema: clinical diagnosis and autopsy findings. An. Bras. Dermatol. [online]. 2016, vol.91, n.5, suppl.1, pp.48-50. ISSN 0365-0596.  http://dx.doi.org/10.1590/abd1806-4841.20164527.

Scleromyxedema is a rare chronic cutaneous mucinosis of unknown etiology. It is characterized by papular eruption and scleroderma with microscopic evidence of mucin deposition, fibroblast proliferation, and fibrosis. Most patients with scleromyxedema have monoclonal gammopathy and systemic manifestations resulting in significant morbidity and mortality. Several types of treatment have been reported with partial or inconsistent responses. Despite showing unpredictable evolution, systemic consequences of scleromyxedema and treatment side effects may result in death. We describe a rare case of a patient with scleromyxedema without paraproteinemia with systemic involvement that evolved to death despite treatment with cyclophosphamide.

Keywords : Autopsy; Cyclophosphamide; Paraproteinemias; Scleromyxedema.

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